Clinical Analysis Of 3 Cases Of Systemic Lupus Erythematosus With Macrophage Activation Syndrome

Objective:To investigate the pathogenesis and clinical characteristic and therapy of patients with systemic lupus erythematosus(SLE) and macrophage activation syndrome(MAS).Methods:The clinical data of 3 cases of SLE with MAS were retrospectively analyzed.Results:None of them had the family medical history of SLE with MAS.All of them had fever,and the main manifestations were hepatomegaly,splenomegalia,lymphadenectasis, hypocytomsis,and various degrees of hepatic lesion.Hemophagocytes were found over 2%in bone marrow slides.After the treatment with glucocorticoid,immunodepressant and combined therapy,2 cases cured,and 1 case died.Conclusion:The diagnosis of MAS is generally not difficult on the basis of clinical manifestati and laboratory examination.Glucocorticoid and immunodepressant may improve symptom and remit pathogenic conditions.The diversity about the level of serum ferritin and the platelet count could be signals as the activity of systemic lupus erythematosus with macrophage activation syndrome.When hypocytomsis suddenly happen,heighten up the level of serum ferritin or jaundice that can't explain with the activity of SLE,it's better to do bone marrow or spleen puncture examination.